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Neurologic and ocular involvements in lyme borreliosis are being increasingly seen in neuro-ophthalmologic practice. This article reviews the specific clinical patterns encountered in the past three years at the Bascom Palmer Eye Institute. Diagnostic workup and therapeutic regimens are recommended.

Clinical data and fundus fluorescein angiograms were analyzed from 35 patients with acute (onset less than four weeks) anterior ischemic optic neuropathy. Nineteen of the 35 patients (54%) had nonarteritic disease, and 16 patients (46%) had giant cell arteritis confirmed by biopsy. Patients with arteritis had higher erythrocyte sedimentation rates, larger cup/disk ratios, and delayed fluorescein dye appearance and choroidal filling times. Three additional patients with cranial arteritis confirmed by biopsy, but without visual loss, had angiographic characteristics similar to patients with arteritic ischemic neuropathy. We consider fluorescein angiography a valuable diagnostic adjunct in identifying patients with giant cell arteritis.

A 2,144-g white girl was born with absence of the right ear and eye, cleft lip and palate, two basal encephaloceles, tricuspid atresia, ventricualr and atrial septal defects, detransposition of the great vessels, right aortic arch, and aberrant right subclavian artery. Through an oval defect in the center of the sphenoid bone, soft tissue protruded into the right nasopharynx. The medial portions of the roof of both orbits and the cribriform plate were absent and soft tissue protruded through this bony defect. Basal tomography was required to demonstrate the encephaloceles, which should be suspected in any child with a median cleft syndrome, a flat broad nasal root, and hypertelorism.

The choroidal circulation completely separated from the retinal circulation has not, to our knowledge, been previously demonstrated by fluorescein angiography. This has been accomplished in owl monkeys and rabbits by direct injection of fluorescein via a catheter introduced into a vortex vein by microsurgical technique. Morphologically distinct choroidal venous and arterial patterns were angiographically demonstrated in monkeys and rabbits. Some direct perfusion to the optic disc from the adjacent choroidal circulation was demonstrated in both species.

In six patients with ocular Lyme borreliosis, bilateral granulomatous iridocyclitis and vitritis were present in five. One of these five also had bilateral optic neuritis. Another patient developed combined trochlear and facial nerve palsies. A syndrome resembling pars planitis with atypical features such as granulomatous keratic precipitates and posterior synechiae should prompt a search for Lyme borreliosis. Topical corticosteroid therapy is necessary to prevent complications of anterior segment inflammation caused by Lyme uveitis, but the benefit of systemic and periocular corticosteroids is uncertain. Oral antibiotics may be effective in treating early stages of ocular Lyme borreliosis. In later stages, intravenous antibiotic therapy is indicated.

A 32-year-old man developed a recurrent nonrhegmatogenous retinal detachment, uveal effusion, and visual loss as a result of latent secondary syphilis. Treponemes were found in the subretinal fluid and the Treponema pallidum hemagglutination test demonstrated substantially higher titers in the subretinal fluid than in the serum (1: 2,569 vs 1: 16). Despite scleral dissection and a scleral implant and treatment with penicillin, the patient's visual loss persisted and the last examination showed a thickened choroid, a flat nonrhegmatogenous retinal detachment, and reaccumulation of subretinal fluid.

Thirteen giant aneurysms in ten patients were retrospectively reviewed. Careful visual field testing of both peripheral and central fields revealed an ipsilateral nasal scotoma in 7 of the 13 aneurysms (54%), or six of the ten patients. A review of 47 giant carotid aneurysms reported in the literature revealed 16 ipsilateral nasal scotomas (34%). The more common intraocular causes of ipsilateral nasal field defects were excluded by careful funduscopic examination. In the absence of intraocular pathologic characteristics, an ipsilateral nasal junction scotoma should alert the clinician to suspect the presence of an otherwise silent internal carotid artery aneurysm.

Three patients presented with typical orbital mucoceles. However, subsequent histopathologic study revealed paranasal sinus concomitant squamous cell carcinoma in all three cases. Careful histologic investigation is necessary before considering orbital mucocele a benign process.

This is a report of the occurrence of nystagmus retractorius, inferior quadrantanopia of optic tract or geniculate origin, and paresis of conjugate gaze, following localized stereotactic lesions in the central nervous system of man. These ocular phenomena were observed after the lesions were placed in the region of the pallidum or thalamus for the relief of tremor and rigidity in extrapyramidal diseases and for the relief of intractable pain. Report of Cases Case 1. —This 72-year-old white woman developed intractable postherpetic neuralgia following herpes zoster ophthalmicus on the right in August, 1958. Protamine, x-ray therapy, avulsion of the right supraorbital nerve, and undermining of the affected skin failed to produce relief. Because of persistence of the pain, she was admitted to Duke Hospital in July, 1960, for stereotactic thalamotomy. Neurological examination revealed a complete anesthesia in the distribution of the first division of the right trigeminal nerve; otherwise, it was

The Pierre Robin syndrome1-4is characterized by three defects: (1) micrognathia, (2) cleft palate, and (3) glossoptosis. This clinical entity is readily apparent at birth as the newborn has immediate difficulty in breathing and nursing. Nasal regurgitation of milk is often noted. Frequent suction and careful nursing care are essential to prevent aspiration in these infants. However, if these children can be maintained until age 5 or 6 years, virtually normal swallowing function will usually develop. After encountering a 2-month-old white boy with Pierre Robin syndrome and severe bilateral congenital glaucoma, our attention was directed to ocular involvement in these cases. This is a report of seven cases of Pierre Robin syndrome, four of whom had bilateral ocular disease. To our knowledge, ocular involvement is a hitherto unrecognized part of this clinical picture. Report of Cases Case 1. —This 2-year-old white boy weighed 5 lb. 7 oz. at birth

Thirty-nine cases of the Pierre Robin syndrome have been reviewed. In one-fourth of the cases, a history of an intrauterine disturbance in early pregnancy was noted. The entity appears to occur more frequently in offspring of elderly mothers. A positive family history was noted in two instances. The history is typical, with difficulty in swallowing and recurrent cyanotic episodes noted in the newborn. The child often reveals marked sternal retraction, excessive mucous secretion and stertorous respirations which appear quite alarming when in the supine position, but there is usually marked improvement when the child is placed in the prone position, allowing the ptotic tongue to fall forward. Physical examination reveals micrognathia (producing an "Andy Gump" appearance), cleft palate or other defects of the hard and soft palate, and various anomalies of the tongue, the most common being glossoptosis. The associated defects are discussed. In the majority of cases the Pierre Robin syndrome is seen without other significant defects than the triad of micrognathia, cleft palate and glossoptosis. Medical and nursing care are considered. Attention is drawn to the occurrence of ocular disease in these children, e.g., congenital glaucoma and retinal detachment. It is recommended that all cases of the Pierre Robin syndrome have a thorough ophthalmologic examination under anesthesia, including dilatation for ophthalmoscopy and tonometry, prior to 1 year of age.

We reviewed eight cases of congenital homonymous hemianopia to emphasize the features that may help the clinician to diagnose a congenital disease. The patients were usually unaware of the visual field defect. The hemianopia was usually congruous when incomplete and the opticokinetic nystagmus had a normal pattern. Associated congenital ocular and neurologic abnormalities were common. Clinically recognizable atrophy or hypoplasia of the optic disk was found in all but one of the patients. There was often a history of neonatal or gestational difficulties followed by emotional, behavioral, and learning problems without mental retardation.

Drusen or hyaline bodies of the optic papilla may be responsible for defects in the visual fields. 1 The occurrence of drusen of the optic nerve in association with an intracranial tumor, however, is extremely rare, only one such case having been reported. 2 For these reasons, the recognition of hyaline bodies in the presence of field defects has been said to obviate the need for further investigation of intracranial structures in some cases. 3 The necessity for diagnostic discretion in such instances has recently been brought to our attention by a patient harboring both hyaline bodies of the optic papillae and a proved pituitary tumor. This is the second reported case to our knowledge of concomitant drusen of the optic nerve and intracranial tumor. Report of Case This patient was seen through the courtesy of Dr. Bernard Levy. This 28-year-old white woman was admitted to Duke Hospital Jan. 18

W. M., a 54-year-old, right-handed white man, was seen because of diplopia on July 18, 1966, through the courtesy of Dr. DeNote. While in col­ lege at the age of 18 years, the patient began to sleep during lectures and would take a nap at lunch daily. Irresistible sleep attacks became pro­ gressively more frequent. Numerous physicians were consulted but his complaints were considered functional. Eventually he would go to sleep even during a telephone conversation or with food in his mouth. Later he began to note diplopia, just prior to a sleep attack. At the age of 29 years, the patient began to experience an unusual reading problem. Just be­ fore a sleep attack, the subject matter would im­ perceptibly change while reading. Thus, the sports page of the newspaper would become phi­ losophy or poetry. By the age of 44 years, color aberrations were noted when reading. Just pre­ ceding a sleep attack, black print often looked red or, less often, green. Within the past year, the patient volunteered that he had seen color televi­ sion on his black and white set. As a prelude to sleep, the black and white picture on the televi­ sion screen would frequently appear a mixture of three colors—red, green, and a variable, poorly described as an intermediate shade of yellow. This had prompted him to question his wife about the remarkable color reception they were obtaining on television, without the expense of buying a color set, but she saw only black and white. At no time were the colors he perceived inappropriate, for example, he had not seen red grass. Sleep paralysis developed at the age of 34 years. These episodes were often preceded by flashing lights, which lasted for a few seconds or minutes and disappeared suddenly. The patient re­ mained conscious during these attacks but could

Superior oblique myokymia is an unusual disorder of ocular motility characterized by rapid, small amplitude, rotary oscillations limited to one eye. Symptoms of monocular oscillopsia and torsional diplopia are experienced by the patient. Although symptomatically obtrusive, the syndrome is benign in our experience. While the cause remains obscure, we have found carbamazepine (Tegretol) to be effective in treating this condition.

A 46-year-old previously healthy man awoke with a profound loss of vision in the left eye. Shortly thereafter, he developed a generalized skin rash. Examination revealed the findings of a combined occlusion of central retinal vein and artery in the left eye, with cutaneous lesions resembling acute erythema nodosum. The cause was proven to be early infectious (secondary) syphilis, and strongly reactive Treponema pallidum immobilization and fluorescent treponemal antibody absorption (FTA-ABS) tests were found in both peripheral blood and aqueous humor of the involved eye. A treponemal organism was found by silver stain in the aqueous humor by Prof Pierre Collart five months after the patient had received a course of 12,000,000 units of parenteral penicillin.

The corneomandibular reflex, or von Solder phenomenon, is an automatic, involuntary movement of the mandible elicited by touching the cornea. This phenomenon is helpful in diagnosis of supranculear lesions of the trigeminal nerve. It simply requires observation of the relaxed and slightly opened jaw when the cornea is stroked with a cotton wisp, and it can be handily included in the testing of corneal sensation. A prominent deviation of the jaw, the result of homolateral contraction of the external pterygoid muscle, constitutes a positive response. Because this reflex has not been previously reported in the ophthalmic literature to our knowledge, the following cases are presented. Report of Cases Case 1. —This 61-year-old Negro male was seen in October, 1961. He had known severe hypertensive vascular disease for many years. He suffered a sudden right hemiplegia in 1952 and a left hemiparesis in 1955. Three weeks prior to admission, he developed

Tic douloureux is generally treated by the neurologist, but frequently is seen by the ophthalmologist because of involvement of the first division of the trigeminal nerve. Blom recently reported rather striking success in a series of 40 cases of tic doul­ oureux followed over six months with a new drug designated as G-32883 or Tegretol. This agent (5-carbamyldibenz-(6,F)-azepin) is an iminostilbene derivative, related to imipramine hydrochloride (Tofranil). Tegretol has an anticonvulsant effect which has been espe­ cially noted with electrically induced convul­ sions in animals. It affects both bulbar and spinal reflexes, particularly polysynaptic reflexes. Spinal monosynaptic reflexes are either unaffected or may be slightly en­ hanced. It is said to be nonnarcotic, to pos­ sess low toxicity and to have a high thera­ peutic index. A common feature of this drug noted by others has been its mood-ele­ vating effect in epileptic patients. Blom's re­ port prompted further investigation of this agent, and the findings in a series of 20 additional patients treated with this drug are here presented.

Two patients had ocular myasthenia gravis coexistent with Hashimoto's thyroiditis. A 37-year-old woman presented with diplopia and displayed a head tremor and thyroid enlargement. Thyroid function tests showed an elevated thyroid stimulating hormone (TSH). A quantitative Tensilon test yeilded a "perverse" response and a needle biopsy indicated Hashimoto's disease. A 26-year-old woman presented with diplopia and subsequently developed blepharoptosis and thyroid enlargement. Examination revealed Cogan's eyelid twitch sign, a paradoxical quantitative Tensilon test, and laboratory studies revealed normal thyroid function tests. Treatment was directed at each disease entity separately. Ocular myasthenia gravis was managed with an anticholinesterase agent in combination with oral corticosteroids that provided additional control. Suppressive therapy with desiccated thyroid hormone reduced the size of the thyroid gland, diminished the signs and symptoms of hypothyroidism, and lowered the levels of TSH, possibly decreasing the risk of thyroid carcinoma. Both patients showed gratifying responses to therapy.

• A young adult who presents with periodic vertigo, diplopia, and tinnitus, later followed by progressive ataxia, may not have multiple sclerosis as might initially be suspected, but rather may represent a type of familial spinocerebellar degeneration previously described by Farmer and Mustian 1 as "vestibulocerebellar ataxia." We recently encountered a patient who presented with these symptoms, and who was found to have downbeat nystagmus, ocular dysmetria, skew deviation, optokinetic dissociation, and a vertical gain bias on horizontal eye movements. Although family involvement was specifically denied by the patient, seven members of her family representing four generations were examined, and found to have similar findings. The importance of a careful neuro-ophthalmologic examination in each available family member of any patient presenting with this constellation of symptoms is emphasized. A discussion of the spinocerebellar degenerations, differential diagnosis, and literature review are included.

• Experience in three cases has shown that a small meningioma in the extreme posterior portion of the orbit just anterior to the optic foramen may occur with progressive visual loss in the absence of orbital signs. Such lesions cannot be detected by plain skull roentgenography, polytomography, or selective angiography with subtraction and magnification. In one patient, even computerized axial tomography gave normal results, and the meningioma went undetected until exploratory craniotomy.